Suspected autoimmune vasculitis with a genetic
Ulcerations on the mucosa of the lower lip of a patient with Behçet disease.
Vasculitis with thrombosis leads to segmental hemorrhagic infarction of
the ear in a patient with Behçet disease.
First described in 1937 by Dr. Helusi Behçet, a
Prevalence is highest in Turkey (80-320:100,000 in the
general population) and Asia (2-30:100,000 in the general population), but
patients from many parts of the world have been described. Male gender is
predominant, especially in severe Behçet disease.
The majority of cases are sporadic, but
familial forms are described. Behçet disease has been associated with
HLA-B51 in 45 to 60% of cases, but the significance of this finding for
the pathogenesis of Behçet disease is not established.
The cornerstone of the disease is a multisystemic
vasculitis involving large and small vessels in various degrees. The
underlying cause has not been defined, but an autoimmune pathogenesis,
including an abnormal T-cell response to microbial antigens and
cross-reaction toward body tissue, is suspected. Characteristically, skin
pathergy is present or so-called positive, that is, an exaggerated reaction
with formation of a papule or a pustula after minor trauma such as a needle
puncture occurs. Although helpful (but not pathognomonic) for the diagnosis of
Behçet disease, the majority of patients do not show this reaction.
No specific tests are available. The diagnosis relies on
the clinical picture, and criteria for establishing the diagnosis have been
published by the International Study Group for Behçet's Disease.
Recurrent oral and genital ulcerations and
uveitis or retinal vasculitis are the characteristic findings in Behçet
disease. Systemic manifestation may affect joints, gut, kidney, lung, and
the cardiovascular system. Lung involvement presents with pulmonary
infarction, hemoptysis, recurrent pneumonia, bronchiolitis obliterans,
organizing pneumonia, and pleurisy. Cardiovascular manifestations include
pulmonary artery aneurysms, intracardiac thrombus formation with right-sided
predominance, and deep venous thrombosis of large veins. Aneurysmatic
changes and thrombosis have been described in many other sites, including
gut and brain. The clinical course of Behçet disease is characterized by
alternating periods of disease activity and remission. Therapy mainly
consists of antiinflammatory and/or immunosuppressive therapy, usually
including steroids with or without cyclophosphamide. Because of the risks
and benefits, the therapeutic approach to clot formation must be evaluated
individually. Mortality is low and usually is the consequence of a
thrombotic or hemorrhagic complication.
The possibility of systemic
involvement has a significant impact on anesthesia. Chest radiography should
be ordered to assess the extent of pulmonary manifestations. Cardiac and
large-vessel involvement can be evaluated sonographically and should include
the potential sites ...